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炎性肌纤维母细胞瘤:一种鉴别诊断范围广泛的罕见实体。

Inflammatory myofibroblastic tumour: a rare entity with wide differential diagnosis.

作者信息

Gilani S M, Kowalski P J

出版信息

Pathologica. 2014 Mar;106(1):1-6.

Abstract

Inflammatory myofibroblastic tumour (IMT) is a rare, distinctive mesenchymal neoplasm. Grossly, it appears as a circumscribed mass with a rubbery to firm cut surface. Microscopically, it is characterized by a spindle cell proliferation within a myxoid stroma with admixed plasma cells, lymphocytes and eosinophils. Immunohistochemical staining is usually positive for vimentin, smooth muscle actin (SMA) and anaplastic lymphoma kinase (ALK). ALK gene rearrangement is present in approximately 50-70% IMTs. The standard treatment is surgical resection, and it is essential to differentiate IMT from benign and malignant mimickers so that appropriate therapy may be provided. Clinical and radiological follow-up is required to detect recurrence.

摘要

炎性肌纤维母细胞瘤(IMT)是一种罕见的、独特的间叶性肿瘤。大体上,它表现为一个边界清楚的肿块,切面呈橡胶样至坚实状。显微镜下,其特征是在黏液样基质内有梭形细胞增生,并伴有浆细胞、淋巴细胞和嗜酸性粒细胞。免疫组化染色通常波形蛋白、平滑肌肌动蛋白(SMA)和间变性淋巴瘤激酶(ALK)呈阳性。约50%-70%的IMT存在ALK基因重排。标准治疗方法是手术切除,必须将IMT与良性和恶性的相似病变区分开来,以便提供适当的治疗。需要进行临床和影像学随访以检测复发情况。

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